Publications by authors named "Judith Therrien"

66 Publications

Neurocognitive disorders amongst patients with congenital heart disease undergoing procedures in childhood.

Int J Cardiol 2021 May 6. Epub 2021 May 6.

McGill Adult Unit for Congenital Heart Disease Excellence, McGill University, Montreal, QC, Canada. Electronic address:

Background: Amongst children with congenital heart disease (CHD), earlier age of repair improves cardiovascular outcomes. The effects of early intervention on neurodevelopment remains unclear. We assessed the association between early life repair, cardiopulmonary bypass (CPB) and the incidence of neurocognitive disorders (NCD) amongst CHD patients.

Methods: We created two retrospective cohorts from the Quebec CHD Database; with data from 1988 to 2010. Complexity of reparative procedures for CHD lesions were used as the proxy of CPB exposure with more complex procedure means longer exposure to CPB. Study Population 1 included pediatric patients with a single reparative procedure and compared patients with complex (long CBP) versus isolated shunt (short CBP) lesions. To assess the effects of CPB alone in Study Population 2 we compared patients with isolated atrial septal defects (ASD) who had surgical (short CBP) versus percutaneous (no CBP) repairs. The primary endpoint for both cohorts was development of an NCD.

Results: In Study population 1, 1174 patients underwent complex surgical repair and 1620 had a shunt closure. The incidence of NCDs was 2.45/100 person-years in the complex surgery group, and 2.08/100 person-years in the shunt closure group. The following were associated with increased risk of developing a NCD: surgical complexity (Hazard Ratio [HR] = 1.21, 95% Confidence Interval [CI]: 1.02-1.44), younger age at intervention (AAI) (HR = 1.21, 95% CI: 1.16-1.25), male sex (HR = 1.88, 95% CI: 1.58-2.23), and later calendar year at intervention (HR = 1.77, 95% CI: 1.05-2.13). Study population 2 had 527 isolated ASD patients; 202 underwent surgical repair and 325 had percutaneous closure. The incidence of NCDs was not statistically different between groups. Male sex (HR = 1.91, 95% CI: 1.61-2.27) and younger AAI (HR = 1.20, 95% CI: 1.16-1.25) were associated with increased NCD risk.

Conclusion: Increased surgical complexity, male sex and younger AAI were associated with increased risk of NCDs in pediatric CHD patients. Surveillance protocols should be considered to identify NCDs in CHD patients after cardiac intervention.
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http://dx.doi.org/10.1016/j.ijcard.2021.05.001DOI Listing
May 2021

Sarcopenia in Fontan patients: a sign of frailty-associated premature ageing?

Cardiol Young 2021 May 6:1-3. Epub 2021 May 6.

McGill Adult Unit for Congenital Heart Disease Excellence, McGill University Health Centre, Montreal, Quebec, Canada.

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http://dx.doi.org/10.1017/S1047951121001748DOI Listing
May 2021

Risk prediction models for heart failure admissions in adults with congenital heart disease.

Int J Cardiol 2021 Jan 14;322:149-157. Epub 2020 Aug 14.

McGill Adult Unit for Congenital Heart Disease Excellence, Montréal, Québec, Canada; iCACH Net Group (Canadian Adult Congenital Heart Disease Network Investigators' Group), Canada. Electronic address:

Background: Heart failure (HF) is the leading cause of death in adult patients with congenital heart disease (ACHD). No risk prediction model exists for HF hospitalization (HFH) for ACHD patients. We aimed to develop a clinically relevant one-year risk prediction system to identify ACHD patients at high risk for HFH.

Methods: Data source was the Quebec CHD Database. A retrospective cohort including all ACHD patients aged 18-64 (1995-2010) was constructed for assessing the cumulative risk of HFH adjusting for competing risk of death. To identify one-year predictors of incident HFH, multivariable logistic regressions were employed to a nested case-control sample of all ACHD patients aged 18-64 in 2009. The final model was used to create a risk score system based on adjusted odds ratios.

Results: The cohort included 29,991 ACHD patients followed for 648,457 person-years. The cumulative HFH risk by age 65 was 12.58%. The case-control sample comprised 26,420 subjects, of whom 189 had HFHs. Significant one-year predictors were age ≥ 50, male sex, CHD lesion severity, recent 12-month HFH history, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes mellitus. The created risk score ranged from 0 to 19. The corresponding HFH risk rose rapidly beyond a score of 8. The risk scoring system demonstrated excellent prediction performance.

Conclusions: One eighth of ACHD population experienced HFH before age 65. Age, sex, CHD lesion severity, recent 12-month HFH history, and comorbidities constructed a risk prediction model that successfully identified patients at high risk for HFH.
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http://dx.doi.org/10.1016/j.ijcard.2020.08.039DOI Listing
January 2021

Echocardiographic Strain Imaging in the Systemic Right Ventricle: Early Clue for Late Decompensation.

Can J Cardiol 2020 09 20;36(9):1341-1343. Epub 2020 Mar 20.

Division of Cardiology, Jewish General Hospital, McGill University, Montreal, Québec, Canada.

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http://dx.doi.org/10.1016/j.cjca.2020.03.013DOI Listing
September 2020

Pulmonary Valve Replacement for Pulmonary Regurgitation in Adults With Tetralogy of Fallot: A Meta-analysis-A Report for the Writing Committee of the 2019 Update of the Canadian Cardiovascular Society Guidelines for the Management of Adults With Congenital Heart Disease.

Can J Cardiol 2019 12 29;35(12):1772-1783. Epub 2019 Aug 29.

McGill Adult Unit for Congenital Heart Disease (MAUDE Unit), McGill University, Montréal, Québec, Canada.

Background: There is no systematic evidence review of the long-term results of surgical pulmonary valve replacement (PVR) dedicated to adults with repaired tetralogy of Fallot (rTOF) and pulmonary regurgitation.

Methods: Our primary objective was to determine whether PVR reduced long-term mortality in adults with rTOF compared with conservative therapy. Secondary objectives were to determine the postoperative incidence rate of death, the changes in functional capacity and in right ventricular (RV) volumes and ejection fraction after PVR, and the postoperative incidence rate of sustained ventricular arrhythmias. A systematic search of multiple databases for studies was conducted without limits.

Results: No eligible randomized controlled trial or cohort study compared outcomes of PVR and conservative therapy in adults with rTOF. We selected 10 cohort studies (total 657 patients) reporting secondary outcomes. After PVR, the pooled incidence rate of death was 1% per year (95% confidence interval [CI] 0-1% per year) and the pooled incidence rate of sustained ventricular arrhythmias was 1% per year (95% CI 1%-2% per year). PVR improved symptoms (odds ratio for postoperative New York Heart Association functional class > II 0.08, 95% CI 0.03-0.24). Indexed RV end-diastolic (-61.29 mL/m, -43.64 to -78.94 mL/m) and end-systolic (-37.20 mL/m, -25.58 to -48.82 mL/m) volumes decreased after PVR, but RV ejection fraction did not change (0.19%, -2.36% to 2.74%). The effect of PVR on RV volumes remained constant regardless of functional status.

Conclusion: Studies comparing PVR and conservative therapy exclusively in adults with rTOF are lacking. After PVR, the incidence rates of death and ventricular tachycardia are both 1 per 100 patient-years. Pooled analyses demonstrated an improved functional status and a reduction in RV volumes.
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http://dx.doi.org/10.1016/j.cjca.2019.08.031DOI Listing
December 2019

Cancer Risk in Congenital Heart Disease-What Is the Evidence?

Can J Cardiol 2019 Dec 9;35(12):1750-1761. Epub 2019 Oct 9.

McGill Adult Unit for Congenital Heart Disease Excellence, Montréal, Québec, Canada. Electronic address:

As life expectancy in patients with congenital heart disease (CHD) has improved, the risk for developing noncardiac morbidities is increasing in adult patients with CHD (ACHD). Among these noncardiac complications, malignancies significantly contribute to the disease burden of ACHD patients. Epidemiologic studies of cancer risk in CHD patients are challenging because they require large numbers of patients, extended follow-up, detailed and validated clinical data, and appropriate reference populations. However, several observational studies suggest that cancer risks are significantly elevated in patients with CHD compared with the general population. CHD and cancer share genetic and environmental risk factors. An association with exposure to low-dose ionizing radiation secondary to medical therapeutic or diagnostic procedures has been reported. Patients with Down syndrome, as well as, to a lesser extent, deletion of 22q11.2 and renin-angiotensin system pathologies, may manifest both CHD and a predisposition to cancer. Such observations suggest that carcinogenesis and CHD may share a common basis in some cases. Finally, specific conditions, such as Fontan circulation and cyanotic CHD, may lead to multisystem consequences and subsequently to cancer. Nonetheless, there is currently no clear consensus regarding appropriate screening for cancer and surveillance modalities in CHD patients. Physicians caring for patients with CHD should be aware of this potential predisposition and meet screening recommendations for the general population fastidiously. An interdisciplinary and global approach is required to bridge the knowledge gap in this field.
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http://dx.doi.org/10.1016/j.cjca.2019.09.023DOI Listing
December 2019

Secular trends in pregnancy rates, delivery outcomes, and related health care utilization among women with congenital heart disease.

Congenit Heart Dis 2019 Sep 17;14(5):735-744. Epub 2019 Jun 17.

McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit), McGill University Health Center, Montreal, Quebec, Canada.

Background: The number of women with congenital heart disease (CHD) of reproductive age is increasing, yet a description of trends in pregnancy and delivery outcomes in this population is lacking.

Objective: To assess secular trends in pregnancy rates, delivery outcomes, and related health care utilization in the adult female CHD population in Quebec, Canada.

Methods: The Quebec CHD database was used to construct a cohort with all women with CHD aged 18-45 years between 1992 and 2004. Pregnancy and delivery rates were determined yearly and compared to the general population. Secular trends in pregnancy and delivery rates were assessed with linear regression. The cesarean delivery rate in the CHD population was compared to the general population. Predictors of cesarean section were determined with multivariable logistic regression. Cox regression, adjusted for comorbidities, was used to analyze the impact of cesarean sections on 1-year health care use following delivery.

Results: About 14 878 women were included. A total of 10 809 pregnancies were identified in 5641 women, of whom 4551 (80%) and 2528 (45%) experienced at least one delivery and/or abortion, respectively. Absolute yearly numbers and rates of pregnancies and deliveries increased during the study period (P < .05). The increment in cesarean section rates was more pronounced among women with CHD than among the general population. Gestational diabetes (OR 1.50, 95% CI [1.13, 1.99]), gestational hypertension (OR 1.81, 95% CI [1.27, 2.57]), and preeclampsia (OR 1.59, 95% CI [1.11, 2.8]) were independent predictors of cesarean delivery. Cesarean sections were associated with postpartum cardiac-hospitalization within 1 year following delivery (HR = 2.35, 95% CI [1.05, 5.28]).

Conclusions: Yearly numbers and rates of pregnancies and deliveries in adult females with CHD rose significantly during the study period. Cesarean sections led to increased health care utilization. Further research is required to determine causes of high cesarean section rates in this patient population.
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http://dx.doi.org/10.1111/chd.12811DOI Listing
September 2019

Maternal Complications and Pregnancy Outcomes After RVOT Reconstruction With an Allograft Conduit.

Authors:
Judith Therrien

J Am Coll Cardiol 2018 06;71(23):2666-2667

Department of Medicine, MAUDE Unit, Sir Mortimer B. Davis Jewish General Hospital and McGill University Health Center, McGill University, Montreal, Quebec, Canada. Electronic address:

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http://dx.doi.org/10.1016/j.jacc.2018.04.007DOI Listing
June 2018

Transesophageal echocardiographic strain imaging predicts aortic biomechanics: Beyond diameter.

J Thorac Cardiovasc Surg 2018 08 11;156(2):503-512.e1. Epub 2018 Mar 11.

Department of Surgery, Royal Victoria Hospital, McGill University, Montreal, Quebec, Canada. Electronic address:

Background: Clinical guidelines recommend resection of ascending aortic aneurysms at diameters 5.5 cm or greater to prevent rupture or dissection. However, approximately 40% of all ascending aortic dissections occur below this threshold. We propose new transesophageal echocardiography strain-imaging moduli coupled with blood pressure measurements to predict aortic dysfunction below the surgical threshold.

Methods: A total of 21 patients undergoing aortic resection were recruited to participate in this study. Transesophageal echocardiography imaging of the aortic short-axis and invasive radial blood pressure traces were taken for 3 cardiac cycles. By using EchoPAC (GE Healthcare, Madison, Wis) and postprocessing in MATLAB (MathWorks, Natick, Mass), circumferential stretch profiles were generated and combined with the blood pressure traces. From these data, 2 in vivo stiffness moduli were calculated: the Cardiac Cycle Pressure Modulus and Cardiac Cycle Stress Modulus. From the resected aortic ring, testing squares were isolated for ex vivo mechanical analysis and histopathology. Each square underwent equibiaxial tensile testing to generate stress-stretch profiles for each patient. Two ex vivo indices were calculated from these profiles (energy loss and incremental stiffness) for comparison with the Cardiac Cycle Pressure Modulus and Cardiac Cycle Stress Modulus.

Results: The echo-derived stiffness moduli demonstrate positive significant covariance with ex vivo tensile biomechanical indices: energy loss (vs Cardiac Cycle Pressure Modulus: R = 0.5873, P < .0001; vs Cardiac Cycle Stress Modulus: R = 0.6401, P < .0001) and apparent stiffness (vs Cardiac Cycle Pressure Modulus: R = 0.2079, P = .0378; vs Cardiac Cycle Stress Modulus: R = 0.3575, P = .0042). Likewise, these transesophageal echocardiography-derived moduli are highly predictive of the histopathologic composition of collagen and elastin (collagen/elastin ratio vs Cardiac Cycle Pressure Modulus: R = 0.6165, P < .0001; vs Cardiac Cycle Stress Modulus: R = 0.6037, P < .0001).

Conclusions: Transesophageal echocardiography-derived stiffness moduli correlate strongly with aortic wall biomechanics and histopathology, which demonstrates the added benefit of using simple echocardiography-derived biomechanics to stratify patient populations.
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http://dx.doi.org/10.1016/j.jtcvs.2018.01.107DOI Listing
August 2018

Exposure to Low-Dose Ionizing Radiation From Cardiac Procedures and Malignancy Risk in Adults With Congenital Heart Disease.

Circulation 2018 03 21;137(13):1334-1345. Epub 2017 Dec 21.

McGill Adult Unit for Congenital Heart Disease Excellence, Montreal, Quebec, Canada (S.C., A.L., L.G., J.T., A.J.M.)

Background: Adults with congenital heart disease (CHD) are exposed to increasing amounts of low-dose ionizing radiation (LDIR) from cardiac procedures. Cancer prevalence in this population is higher than in the general population. This study estimates the association between LDIR exposure from cardiac procedures and incident cancer in adult patients with CHD.

Methods: The study population derived from the Quebec Congenital Heart Disease Database. We measured cumulative numbers of LDIR-related cardiac procedures for each patient until 1 year before the time of cancer diagnosis or administrative censoring. To assess the association between LDIR exposure and cancer risk, we conducted a nested case-control study and matched cancer cases with controls on sex, CHD severity, birth year, and age.

Results: The study included 24 833 adult patients with CHD aged 18 to 64 years from 1995 to 2009. In >250 791 person-years of follow-up, 602 cancer cases were observed (median age, 55.4 years). The cumulative incidence of cancer estimated up to 64 years of age was 15.3% (95% confidence interval [CI], 14.2-16.5). Cases had more LDIR-related cardiac procedures than controls (1410 versus 921 per 1000 adult patients with CHD, <0.0001). Cumulative LDIR exposure was independently associated with cancer (odds ratio [OR], 1.08 per procedure; 95% CI, 1.04-1.13). Similar results were obtained by using dose estimates for LDIR exposure (OR, 1.10 per 10 mSv; 95% CI, 1.05-1.15) with a possible dose-related response. The effect measure was in the same direction, and the association was persistent for exposure from ≥6 procedures in all sensitivity analyses: after excluding most smoking-related cancer cases (OR, 1.10 per procedure; 95% CI, 1.05-1.16 and OR when exposure from ≥6 procedures, 3.08; 95% CI, 1.77-5.37), and after applying a 3-year lag period (OR, 1.09 per procedure; 95% CI, 1.03-1.14 and OR when exposure from ≥6 procedures: 2.58; 95% CI, 1.43-4.69).

Conclusions: To our knowledge, this is the first large population-based study to analyze and document the association between LDIR-related cardiac procedures and incident cancer in the population of adults with CHD. Confirmations of these findings by prospective studies are needed to reinforce policy recommendations for radiation surveillance in patients with CHD where no regulation currently exists. Physicians ordering and performing cardiac imaging should ensure that exposure is as low as reasonably achievable without sacrificing quality of care.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.117.029138DOI Listing
March 2018

Repeated blood transfusions: Identification of a novel culprit of early graft failure in children.

Int J Cardiol 2018 03 5;254:90-95. Epub 2017 Dec 5.

McGill Adult Unit for Congenital Heart Disease Excellence, Montreal, Canada.

Background: The attrition of right ventricle to pulmonary artery (RV-PA) grafts has been attributed in part to the body's immunologic response. We hypothesized that antibodies developed through blood transfusion, directed against the grafts, may result in accelerated degeneration and the need for re-intervention.

Methods: This is a population-based study of the province of Quebec. We included children born between January 1, 1987 to December 31, 2006 who were diagnosed with a cono-truncal anomaly and had an RV-PA graft. The patients were followed for transfusion exposure and RV-PA graft re-intervention. Time to re-intervention in those exposed versus non-exposed was analyzed using Cox regression. Analysis was done in two time periods, before and after the calendar year 2000, given the change in blood preparation in the province of Quebec.

Results: There were 413 patients who met the inclusion criteria of a cono-truncal disorder. Of the whole study population, 69% received a blood transfusion. Cox regression analysis showed that among patients who had the initial graft performed (n=181) before year 2000, having 2 or more blood transfusion was associated with an nearly tripled risk of a re-intervention comparing to no blood transfusion (hazard ratio of 2.88; 95% confidence interval 1.05-7.91). In patients who had the initial graft performed after year 2000 (n=232), the associated risk increase was 7-fold (hazard ratio of 7.01; 95% confidence interval 3.06-16.02). Kaplan-Meier analyses confirmed the significant difference in the re-intervention free survival probabilities between those who received 2 or more blood product transfusion and those who did not as well: prior to year 2000 (67.9% vs. 88.0% at 5years, p=0.0201) as well as after year 2000 (39.7% vs. 82.8% at 5years, p<0.0001).

Conclusion: In this population-based analysis, repeated blood product transfusion was associated with a significant increased risk of a need for RV-PA graft re-intervention. This data strongly suggest that repeated blood transfusion may adversely impact graft longevity.
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http://dx.doi.org/10.1016/j.ijcard.2017.11.112DOI Listing
March 2018

Magnetic-guided catheter ablation of twin AV nodal reentrant tachycardia in a patient with left atrial isomerism, interrupted inferior vena cana, and Kawashima-Fontan procedure.

Clin Case Rep 2017 12 13;5(12):2105-2110. Epub 2017 Nov 13.

Montreal Heart Institute Université de Montréal Montreal Quebec Canada.

Twin AV nodal reentrant tachycardia most commonly occurs in patients with complex congenital heart disease who have two distinct AV nodes, His bundles, and non-preexcited QRS morphologies. Catheter ablation of the weaker AV node may be hindered by anatomical complexities. In such cases, remote magnetic guidance offers a potentially effective solution.
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http://dx.doi.org/10.1002/ccr3.1263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5715600PMC
December 2017

Incidence, Predictors, and Mortality of Infective Endocarditis in Adults With Congenital Heart Disease Without Prosthetic Valves.

Am J Cardiol 2017 Dec 14;120(12):2278-2283. Epub 2017 Oct 14.

McGill Adult Unit for Congenital Heart Disease Excellence, McGill University Health Centre, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics and Occupational Health, McGill University, Montreal, Quebec, Canada. Electronic address:

Congenital heart disease (CHD) increases the risk of infective endocarditis (IE), though the lesion-specific risk and mortality are poorly defined. Using the population-based Quebec CHD database, we sought to describe the predictors of IE and to evaluate if IE was associated with mortality among adult CHD (ACHD) patients without prior valve replacement surgery. We extracted data on ACHD patients with IE and assessed the lesion-specific incidence of IE, risk factors for IE acquisition, and all-cause 1-year mortality. Among 29,866 ACHD patients, 285 (0.95%) developed IE during follow-up period of 378,901 patient-years, from 1988-2010. The highest and lowest lesion-specific incidences of IE were observed with left-sided lesions (1.61/1000 patient-years) and patent ductus arteriosus (0.24/1000 patient-years), respectively. The following predicted the risk of IE acquisition (odds ratio (OR), 95% confidence interval [CI]): cardiac surgery in the previous 6 months (9.07, 3.98-20.67), endocardial cushion defects (6.65, 3.84-11.53), left-sided lesions (5.11, 3.60-7.25), cyanosis at birth (4.82, 3.12-7.46), ventricular septal defect (2.81, 1.87-4.21), diabetes mellitus (1.65, 1.10-2.48), and recent medical interventions (12.52, 5.23-29.97). Twenty-five (8.77%) patients died within 1-year of IE diagnosis, a substantially elevated rate compared to patients without IE (OR 31.07, 95%CI 16.23-59.49). The risk of death following IE diagnosis was similarly elevated among patients with left-sided, cyanotic and other CHD lesions. In conclusion, the risk of IE in ACHD patients is lesion-specific and is greatest in the context of recent medical interventions. IE is associated with increased 1-year mortality, irrespective of broad CHD lesion grouping.
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http://dx.doi.org/10.1016/j.amjcard.2017.08.051DOI Listing
December 2017

Cardiac output as a predictor in congenital heart disease: Are we stating the obvious?

Int J Cardiol 2016 May 8;210:143-8. Epub 2016 Feb 8.

McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit), McGill University Health Center, Montréal, Canada; Beth Raby Adult Congenital Heart Disease Clinic, Jewish General Hospital, Montréal, Canada. Electronic address:

Background: Significant pulmonary regurgitation, declining right-sided ejection fraction, increased right ventricular (RV) volumes as well as left ventricular (LV) dysfunction have all been identified as predictors of poor outcomes in patients with congenital heart disease (CHD). The prognostic value of the cardiac output (CO) in these patients however has never been studied.

Methods: All consecutive ambulatory adult patients with CHD referred for magnetic resonance imaging (MRI) at the Montreal Children's Hospital between June 2007 and May 2009 were included. Right ventricular (RV) and left ventricular (LV) variables including end diastolic and end systolic volumes (EDV, ESV respectively), ejection fractions (EF) and regurgitant volumes were obtained. Cardiac index (CI) was calculated. Patients were followed for cardiac-related hospitalizations and cardiac interventions.

Results: Ninety-six patients were included. Median follow up was 3.9 ± 1.4 years. Nineteen percent of patients had a systemic CI<2.4 l/min/m(2). LVEDV, LVEF and RVEF were significantly diminished in the low CI group with a significant increase in RVESV and total regurgitant volume. Best predictors of low CI were LVEF (AUC=0.74), RVEF (AUC=0.71), total RV regurgitant volume (AUC=0.64) and RVESV (AUC=0.563). Low systemic CI was the best predictor of cardiac-related hospitalizations (hazard ratio 3.5, 95% confidence interval 1.5-8.5) and cardiac interventions (hazard ratio 2.2, 95% confidence interval 1.3-4.0), superior to LVEF, RVEF, total regurgitant volume and RVESV parameters.

Conclusions: In patients with congenital heart disease, cardiac index is the best predictor of cardiac hospitalizations and cardiac interventions.
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http://dx.doi.org/10.1016/j.ijcard.2016.02.071DOI Listing
May 2016

Stroke in Adults With Congenital Heart Disease: Incidence, Cumulative Risk, and Predictors.

Circulation 2015 Dec 23;132(25):2385-94. Epub 2015 Nov 23.

From the McGill Adult Unit for Congenital Heart Disease Excellence, Montreal, Québec, Canada (J.L., J.T., M.K., L.G., A.J.M.); Department of Medicine, McGill University Health Centre, Montreal, Québec, Canada (J.M.B., A.J.M.); and Division of Cardiology, Jewish General Hospital, Montreal, Québec, Canada (J.T.).

Background: Stroke is an important cause of morbidity and mortality, although there is a lack of comprehensive data on its incidence, cumulative risk, and predictors in patients with adult congenital heart disease.

Methods And Results: This retrospective study of 29 638 Quebec patients with adult congenital heart disease aged 18 to 64 years between 1998 and 2010 was based on province-wide administrative data. The cumulative risk of ischemic stroke estimated up to age 64 years was 6.1% (95% confidence interval [CI], 5.0-7.0%) in women and 7.7% (95% CI, 6.4-8.8%) in men; the risk of hemorrhagic stroke was 0.8% (95% CI, 0.4-1.2%) and 1.3% (95% CI, 0.8-1.8%), respectively. Compared with rates reported for the general Quebec population, age-sex standardized incidence rates of ischemic stroke were 9 to 12 times higher below age 55 years and 2 to 4 times higher in the age group 55 to 64 years; hemorrhagic stroke rates were 5 to 6 times (age <55 years) and 2 to 3 times higher. Using a combination of stepwise model selection and Bayesian model averaging, the strongest predictors of ischemic stroke were heart failure (odds ratio for age group 18-49 years, 5.94 [95% CI, 3.49-10.14], odds ratio for age group 50-64 years, 1.68 [95% CI, 1.06-2.66]), diabetes mellitus (odds ratio, 2.33 [95% CI, 1.66-3.28]), and recent myocardial infarction (odds ratio, 8.38 [95% CI, 1.77-39.58]).

Conclusions: Among patients with adult congenital heart disease, 1 in 11 men and 1 in 15 women experienced a stroke between ages 18 and 64 years. Stroke incidence was considerably higher than in the general population, especially at a younger age. The most important predictors of ischemic stroke were heart failure, diabetes mellitus, and recent myocardial infarction. Additional research is required to see whether advances in the management of adult congenital heart disease may reduce this substantial stroke rate.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.115.011241DOI Listing
December 2015

Understanding and treating aortopathy in bicuspid aortic valve.

Trends Cardiovasc Med 2015 Jul 17;25(5):445-51. Epub 2014 Dec 17.

Jewish General Hospital, Montreal, Quebec, Canada. Electronic address:

Bicuspid aortic valve is a simple yet common condition with surprising clinical complexity. Patients are typically found to have enlargement of the ascending aorta, either root or midascending, independent of valve function. This enlargement is perceived to be a risk of aortic dissection, and therefore, prophylactic surgery is often recommended when the aorta reaches 50-55 mm in diameter. Despite the prevalence and potential health impact, there is to date no published data on the role of medical therapy. The emerging trend, however, is to address this question through clinical research. Early trials are underway designed to address the question of potential medical therapy. This article reviews current knowledge of the condition, its potential etiology, and ongoing work to assess the role of medical therapy.
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http://dx.doi.org/10.1016/j.tcm.2014.12.006DOI Listing
July 2015

Rationale and design of the Canadian Outcomes Registry Late After Tetralogy of Fallot Repair: the CORRELATE study.

Can J Cardiol 2014 Nov 24;30(11):1436-43. Epub 2014 Jun 24.

Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada.

Background: Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined.

Methods: Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated.

Results: To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults.

Conclusions: CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR.
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http://dx.doi.org/10.1016/j.cjca.2014.06.011DOI Listing
November 2014

Canadian Cardiovascular Society position statement on the management of thoracic aortic disease.

Can J Cardiol 2014 Jun 28;30(6):577-89. Epub 2014 Feb 28.

Division of Cardiology, Western University, London, Ontario, Canada.

This Canadian Cardiovascular Society position statement aims to provide succinct perspectives on key issues in the management of thoracic aortic disease (TAD). This document is not a comprehensive overview of TAD and important elements of the epidemiology, presentation, diagnosis, and management of acute aortic syndromes are deliberately not discussed; readers are referred to the 2010 guidelines published by the American Heart Association, American College of Cardiology, American Association for Thoracic Surgery, and other stakeholders. Rather, this document is a practical guide for clinicians managing adult patients with TAD. Topics covered include size thresholds for surgical intervention, emerging therapies, imaging modalities, medical and lifestyle management, and genetics of TAD. The primary panel consisted of experts from a variety of disciplines that are essential for comprehensive management of TAD patients. The methodology involved a focused literature review with an emphasis on updates since 2010 and the use of Grading of Recommendations Assessment, Development, and Evaluation methodology to arrive at specific recommendations. The final document then underwent review by a secondary panel. This document aims to provide recommendations for most patients and situations. However, the ultimate judgement regarding the management of any individual patients should be made by their health care team.
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http://dx.doi.org/10.1016/j.cjca.2014.02.018DOI Listing
June 2014

Anatomical considerations for the development of a new transcatheter aortopulmonary shunt device in patients with severe pulmonary arterial hypertension.

Pulm Circ 2013 Sep 18;3(3):639-46. Epub 2013 Nov 18.

1 McGill Adult Unit for Congenital Heart Disease Excellence, Royal Victoria Hospital, Montreal, Quebec, Canada.

Abstract Morbidity from pulmonary arterial hypertension (PAH) ensues when the pulmonary pressure reaches suprasystemic levels. A transcatheter alternative to the Potts shunt would allow decompression of the right heart without the surgical risks. To aid development of a transcatheter aortopulmonary shunt (TAPS) device, we described the anatomic relationship between the left pulmonary artery (LPA) and the descending aorta (dAO) in adults with severe pulmonary hypertension. Adults with severe PAH (peak systolic pulmonary arterial pressure [PASP] ≥80 mmHg) who had computed tomography of the chest were enrolled. Measurements were taken on the axial plane at the level of the pulmonary artery bifurcation. Forty patients (male sex, 9 patients; median age ± standard deviation [SD], [Formula: see text] years; median PASP ± SD, [Formula: see text] mmHg) were identified. The mean distance (±SD) between the LPA and dAO was [Formula: see text] mm. The mean luminal dAO and LPA diameters (±SD) were [Formula: see text] mm and [Formula: see text] mm, respectively. The LPA and dAO approximated in 93% of patients, with 38% having aortic calcification at the contact site. The mean "landing zone" width and height (defining an area with distance <4 mm between the outer borders) of the two arteries were [Formula: see text] mm and [Formula: see text] mm, respectively, at a mean distance of [Formula: see text] mm from the main pulmonary artery bifurcation. This study shows that the landing zone is able to accommodate a TAPS device of up to 15 mm in diameter in the majority of patients with severe PAH.
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http://dx.doi.org/10.1086/674328DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4070815PMC
September 2013

Specialized adult congenital heart disease care: the impact of policy on mortality.

Circulation 2014 May 3;129(18):1804-12. Epub 2014 Mar 3.

McGill Adult Unit for Congenital Heart Disease, McGill University Health Centre, Montreal, Canada (D.M., R.I.-I., J.T., A.M.); Department of Epidemiology & Biostatistics, McGill University, Montreal, Canada (L.P., M.A.); Division of General Internal Medicine, Department of Medicine, McGill University, Montreal, Canada (L.P.); Division of Clinical Epidemiology, Research Institute McGill University Health Center, Montreal, Canada (L.P., M.A.); Department of Cardiology, Montreal Heart Institute, Université de Montréal, Montreal, Canada (P.K.); and Stollery Children's Hospital and University of Alberta, Edmonton, Alberta, Canada (A.S.M.).

Background: Clinical guidelines recommend specialized care for adult congenital heart disease (ACHD) patients. In reality, few patients receive such dedicated care. We sought to examine the impact of specialized care on ACHD patient mortality.

Methods And Results: We examined referral rates to specialized ACHD centers and ACHD patient mortality rates between 1990 and 2005 in the population-based Quebec Congenital Heart Disease database (n=71 467). This period covers several years before and after the publication of guidelines endorsing specialized care for ACHD patients. A time-series design, based on Joinpoint and Poisson regression analyses, was used to assess the changes in annual referral and patient mortality rates. The association between specialized ACHD care and all-cause mortality was assessed in both case-control and cohort studies. The time-series analysis demonstrated a significant increase in referral rates to specialized ACHD centers in 1997 (rate ratio, +7.4%; 95% confidence interval [CI], +6.6% to +8.2%). In parallel, a significant reduction in expected ACHD patient mortality was observed after year 2000 (rate ratio, -5.0%;95% CI, -10.8% to -0.8%). In exploratory post hoc cohort and case-control analyses, specialized ACHD care was independently associated with reduced mortality (hazard ratio, 0.78; 95% CI, 0.65-0.94) and a reduced odds of death (adjusted odds ratio, 0.82; 95% CI, 0.08-0.97), respectively. This effect was predominantly driven by patients with severe congenital heart disease (hazard ratio, 0.38; 95% CI, 0.22-0.67).

Conclusions: A significant increase in referrals to specialized ACHD centers followed the introduction of the clinical guidelines. Moreover, referral to specialized ACHD care was independently associated with a significant mortality reduction. Our findings support a model of specialized care for all ACHD patients.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.113.005817DOI Listing
May 2014

Long-term cost-effectiveness of transcatheter versus surgical closure of secundum atrial septal defect in adults.

Int J Cardiol 2014 Mar 8;172(1):109-14. Epub 2014 Jan 8.

McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit), McGill University Health Centre, Canada. Electronic address:

Background: The most common congenital anomaly in adults is secundum, which can be closed using a surgical or transcatheter approach. Despite the growing use of transcatheter ASD closure, few studies have examined the cost-effectiveness of this strategy. We sought to compare the long-term cost effectiveness of transcatheter and surgical closure of secundum in adults.

Methods: A decision-analytic model was used with all clinical outcome parameter estimates obtained from the province-wide Québec Congenital Heart Disease Database. Costs were obtained from a single academic centre (Canadian dollars). A cost-effectiveness analysis using a discrete event Monte Carlo simulation model from the perspective of a single third party payer and multiple sensitivity analyses were performed. Patients were followed for a maximum of 5 years after ASD closure.

Results: Between l998 and 2005, we identified 718 adults (n=335 transcatheter; n=383 surgical) who underwent ASD closure in Quebec. The 5-year cost of surgical closure was $15,304 SD $4581 versus $11,060 SD $5169 for the transcatheter alternative. At 5 years, transcatheter closure was marginally more effective than surgery (4.683 SD 0.379 life-years versus 4.618 SD 0.638 life-years). Probabilistic sensitivity analyses demonstrated that transcatheter ASD closure was a dominant strategy with an 80% probability of cost savings and equal or greater efficacy compared to surgical treatment.

Conclusion: Although definitive conclusions are limited given the observational nature of the primary data sources, transcatheter ASD closure appeared to be a cost-effective strategy associated with slightly improved clinical outcomes and reduced costs compared to surgical closure at 5-years follow-up.
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http://dx.doi.org/10.1016/j.ijcard.2013.12.144DOI Listing
March 2014

Infective endocarditis in children with congenital heart disease: cumulative incidence and predictors.

Circulation 2013 Sep;128(13):1412-9

Department of Epidemiology, Biostatistics and Occupational Health; McGill University, Montreal, Canada (D.R., J.S.K., R.I.-I., L.P., A.J.M.); Division of Cardiology, Stollery Children's Hospital, Edmonton, Canada (A.S.M.); Division of General Internal Medicine, McGill University Health Center, Montreal, Canada (L.P.); and McGill Adult Unit for Congenital Heart Disease, McGill University Health Center, Montreal, Canada (J.T., A.J.M.).

Background: The American Heart Association guidelines for prevention of infective endocarditis (IE) in 2007 reduced the groups of congenital heart disease (CHD) patients for whom antibiotic prophylaxis was indicated. The evidence base in CHD patients is limited. We sought to determine the risk of IE in children with CHD.

Methods And Results: We performed a population-based analysis to determine the cumulative incidence and predictors of IE in children (0-18 years) with CHD by the use of the Quebec CHD Database from 1988 to 2010. In 47 518 children with CHD followed for 458 109 patient-years, 185 cases of IE were observed. Cumulative incidence of IE was estimated in the subset of 34 279 children with CHD followed since birth, in whom the risk of IE up to 18 years of age was 6.1/1000 children (95% confidence interval, 5.0-7.5). In a nested case-control analysis, the following CHD lesions were at highest risk of IE in comparison with atrial septal defects (adjusted rate ratio, 95% confidence interval): cyanotic CHD (6.44, 3.95-10.50), endocardial cushion defects (5.47, 2.89-10.36), and left-sided lesions (1.88, 1.01-3.49). Cardiac surgery within 6 months (5.34, 2.49-11.43) and an age of <3 years (3.53, 2.51-4.96; reference, ages 6-18) also conferred an elevated risk of IE.

Conclusions: In a large population-based cohort of children with CHD, we documented the cumulative incidence of IE and associated factors. These findings help identify groups of patients who are at the highest risk of developing IE.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.113.001827DOI Listing
September 2013

Sex differences in hospital mortality in adults with congenital heart disease: the impact of reproductive health.

J Am Coll Cardiol 2013 Jul 1;62(1):58-67. Epub 2013 May 1.

Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands.

Objectives: The study objectives were to analyze sex differences in hospital mortality of adult patients with congenital heart disease (CHD) and to determine the impact of health services associated with pregnancy on outcomes in women.

Background: The determinants of sex differences in the demographic distribution of CHD are poorly understood.

Methods: The Quebec CHD database and the Dutch CONCOR (CONgenital CORvitia) registry were used to identify patients with CHD aged 18 to 65 years who were hospitalized between 1996 and 2005. Regression analyses were used to compare 30-day in-hospital mortality in men versus women and in women aged 18 to 45 years with versus without a pregnancy history, after adjustment for age, CHD severity, comorbidities, and admission diagnosis.

Results: Of 39,776 patients followed for 259,741 patient years, 19,099 patients (48%) had 54,195 admissions (62% among women). In those aged 18 to 45 years, 30-day in-hospital mortality was higher in men compared with women with nonpregnancy admissions (adjusted rate ratio: 1.36; 95% confidence interval: 1.02 to 1.81). The adjusted rate ratio for 30-day in-hospital mortality in women with a pregnancy history compared with those without was 0.49 (95% confidence interval: 0.24 to 0.99). A history of pregnancy was not associated with an overall increase in medical encounters.

Conclusions: We demonstrated a protective effect of sex on in-hospital mortality in women with CHD of reproductive age that did not correlate with increased medical surveillance. Future studies need to explore other mechanisms to account for our observations. Understanding the determinants of the sex distribution of adults with CHD is important for our ability to predict demographic changes in the population with CHD.
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http://dx.doi.org/10.1016/j.jacc.2013.03.056DOI Listing
July 2013

Long-term outcomes after surgical versus transcatheter closure of atrial septal defects in adults.

JACC Cardiovasc Interv 2013 May 17;6(5):497-503. Epub 2013 Apr 17.

McGill Adult Unit for Congenital Heart Disease Excellence, McGill University Health Centre, Montréal, Québec, Canada.

Objectives: The purpose of this study was to assess the comparative effectiveness and long-term safety of transcatheter versus surgical closure of secundum atrial septal defects (ASD) in adults.

Background: Transcatheter ASD closure has largely replaced surgery in most industrialized countries, but long-term data comparing the 2 techniques are limited.

Methods: We performed a retrospective population-based cohort study of all patients, ages 18 to 75 years, who had surgical or transcatheter ASD closure in Québec, Canada's second-largest province, using provincial administrative databases. Primary outcomes were long-term (5-year) reintervention and all-cause mortality. Secondary outcomes were short-term (1-year) onset of congestive heart failure, stroke, or transient ischemic attack, and markers of health service use.

Results: Of the 718 ASD closures performed between 1988 and 2005, 383 were surgical and 335 were transcatheter. The long-term reintervention rate was higher in patients with transcatheter ASD closure (7.9% vs. 0.3% at 5 years, p = 0.0038), but the majority of these reinterventions occurred in the first year. Long-term mortality with the transcatheter technique was not inferior to surgical ASD closure (5.3% vs. 6.3% at 5 years, p = 1.00). Secondary outcomes were similar in the 2 groups.

Conclusions: Transcatheter ASD closure is associated with a higher long-term reintervention rate and long-term mortality that is not inferior to surgery. Overall, these data support the current practice of using transcatheter ASD closure in the majority of eligible patients and support the decision to intervene on ASD with significant shunts before symptoms become evident.
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http://dx.doi.org/10.1016/j.jcin.2012.12.126DOI Listing
May 2013

Right ventricular end-diastolic wall stress: does it impact on right atrial size, and does it differ in right ventricular pressure vs volume loading conditions?

Can J Cardiol 2013 Jul 11;29(7):858-65. Epub 2012 Oct 11.

MAUDE Unit (McGill University Health Network/Beth Raby Adult Congenital Heart Disease Clinic, Jewish General Hospital), Division of Cardiology, McGill University, Montreal, Québec, Canada.

Background: Right ventricular (RV) diastolic dysfunction precedes RV systolic dysfunction. Improvement in noninvasive assessment of RV diastolic function may enable earlier detection of RV dysfunction, especially important in the assessment of patients with congenital heart disease. We investigated a new parameter we call RV end-diastolic wall stress (RVEDWS) in an effort to better characterize RV diastolic function.

Methods: We retrospectively studied consecutive adults with right-sided congenital heart disease between January 2005 and November 2006. RVEDWS was calculated with the Laplace law: r × p/λ, where r = basal RV dimension at end-diastole, p = RV end-diastolic pressure obtained from catheterization of the right side of the heart, and λ = thickness of RV free wall at end-diastole in the subcostal view. Calculated RVEDWS was correlated to echocardiographically derived right atrial (RA) measurements by means of the Pearson correlation.

Results: Twenty-four patients, aged 41 ± 15 years, were included in the study. Mean RVEDWS was 20 ± 11 g/cm(2) (range, 3-46 g/cm(2)). RVEDWS correlated significantly with RA area and volume (r = 0.71, P < 0.0001; r = 0.69, P < 0.001, respectively). An RVEDWS > 17 g/cm(2) had a sensitivity of 91% and specificity of 85% in predicting significant RA enlargement. RVEDWS was significantly higher in patients with RV volume overload compared with those with pressure or normal loading conditions (28 g/cm(2) vs 17 g/cm(2), P = 0.01).

Conclusions: RVEDWS correlates significantly with RA size and differs considerably between RV volume and pressure overload states. Further work is needed to determine whether this RV diastolic parameter can be predictive of clinical outcomes in patients with RV loading lesions.
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http://dx.doi.org/10.1016/j.cjca.2012.06.024DOI Listing
July 2013

Spot diagnosis using pulse wave Doppler interrogation of the abdominal aorta.

J Cardiovasc Ultrasound 2012 Jun 25;20(2):112-3. Epub 2012 Jun 25.

Division of Cardiology, McGill University Health Center, Montreal, QC, Canada.

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http://dx.doi.org/10.4250/jcu.2012.20.2.112DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3391628PMC
June 2012

Coarctation of the aorta and coronary artery disease: fact or fiction?

Circulation 2012 Jul 6;126(1):16-21. Epub 2012 Jun 6.

McGill Adult Unit for Congenital Heart Disease, McGill University Health Center, Montreal, QC, Canada.

Background: Aortic coarctation (CoA) is reported to predispose to coronary artery disease (CAD). However, our clinical observations do not support this premise. Our objectives were to describe the prevalence of CAD among adults with CoA and to determine whether CoA is an independent predictor of CAD or premature CAD.

Methods And Results: The study population was derived from the Quebec Congenital Heart Disease Database. We compared patients with CoA and those with a ventricular septal defect, who are not known to be at increased risk of CAD. The prevalence of CAD in patients with CoA compared with those with ventricular septal defect was determined. We then used a nested case-control design to determine whether CoA independently predicted for the development of CAD. Of 756 patients with CoA who were alive in 2005, 37 had a history of CAD compared with 224 of 6481 patients with ventricular septal defect (4.9% versus 3.5%; P=0.04). Male sex (odds ratio [OR], 2.13; 95% confidence interval [CI], 1.62-2.80), hypertension (OR, 1.95; 95% CI, 1.44-2.64), diabetes mellitus (OR, 1.68; 95% CI, 1.09-2.58), age (OR per 10-year increase, 2.28; 95% CI, 2.09-2.48), and hyperlipidemia (OR, 11.58; 95% CI, 5.75-23.3) all independently predicted for the development of CAD. CoA did not independently predict for the development of CAD (OR, 1.04; 95% CI, 0.68-1.57) or premature CAD (OR for CoA versus ventricular septal defect, 1.44; 95% CI, 0.79-2.64) after adjustment for other factors.

Conclusions: Although traditional cardiovascular risk factors independently predicted for the development of CAD, the diagnosis of CoA alone did not. Our findings suggest that cardiovascular outcomes of these patients may be improved with tight risk factor control.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.111.088294DOI Listing
July 2012

Mirror image atrial dilatation in adult patients with atrial fibrillation and congenital heart disease.

Int J Cardiol 2013 Aug 26;167(3):816-20. Epub 2012 Feb 26.

McGill Adult Unit for Congenital Heart Disease Excellence, Montreal, Canada.

Background: Atrial fibrillation (AF) is largely regarded to be initiated from left atrial (LA) dilatation, with subsequent dilatation of the right atrium (RA) in those who progress to chronic AF. We hypothesized that in adult patients with right-sided congenital heart disease (CHD) and AF, RA dilatation will predominate with subsequent dilatation of the left atrium, as a mirror image.

Methods: Adult patients with diagnosis of right-sided, ASD or left-sided CHD who had undergone an echocardiographic study and electrocardiographic recording in 2007 were included. RA and LA area were measured from the apical view. AF was diagnosed from a 12-lead electrocardiogram or Holter recording. A multivariate logistic regression model was used to identify predictors of AF and linear regression models were performed to measure relationship between RA and LA area and AF.

Results: A total of 291 patients were included in the study. Multivariate analysis showed that age (p=0.0001), RA (p=0.025) and LA area (p=0.0016) were significantly related to AF. In patients with pure left-sided pathologies, there was progressive and predominant LA dilatation that paralleled the development of AF from none to paroxysmal to chronic AF. In patients with pure right-sided pathologies, there was a mirror image of progressive and predominant RA dilatation with the development of AF.

Conclusion: We observed a mirror image atrial dilatation in patients with right sided disease and AF. This may provide novel mechanistic insight as to the origin of AF in these patients and deserves further studying in the form of targeted electrophysiological studies.
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http://dx.doi.org/10.1016/j.ijcard.2012.02.016DOI Listing
August 2013

Geriatric congenital heart disease: burden of disease and predictors of mortality.

J Am Coll Cardiol 2011 Sep;58(14):1509-15

Department of Medicine, McGill University, Montreal, Quebec, Canada.

Objectives: The study sought to measure the prevalence, disease burden, and determinants of mortality in geriatric adults with congenital heart disease (ACHD).

Background: The population of ACHD is increasing and aging. The geriatric ACHD population has yet to be characterized.

Methods: Population-based cohort study using the Quebec Congenital Heart Disease Database of all patients with congenital heart disease coming into contact with the Quebec healthcare system between 1983 and 2005. Subjects with specific diagnoses of congenital heart disease and age 65 years at time of entry into the cohort were followed for up to 15 years. The primary outcome was all-cause mortality.

Results: The geriatric ACHD cohort consisted of 3,239 patients. From 1990 to 2005, the prevalence of ACHD in older adults remained constant from 3.8 to 3.7 per 1,000 indexed to the general population (prevalence odds ratio: 0.98; 95% confidence interval [CI]: 0.93 to 1.03). The age-stratified population prevalence of ACHD was similar in older and younger adults. The most common types of congenital heart disease lesions in older adults were shunt lesions (60%), followed by valvular lesions (37%) and severe congenital heart lesions (3%). Type of ACHD and ACHD-related complications had a minor impact on mortality, which was predominantly driven by acquired comorbid conditions. The most powerful predictors of mortality in the Cox proportional hazards model were: dementia (hazard ratio [HR]: 3.24; 95% CI: 1.53 to 6.85), gastrointestinal bleed (HR: 2.79; 95% CI: 1.66 to 4.69), and chronic kidney disease (HR: 2.50; 95% CI: 1.72 to 3.65).

Conclusions: The prevalence of geriatric ACHD is substantial, although severe lesions remain uncommon. ACHD patients that live long enough acquire general medical comorbidities, which are the pre-eminent determinants of their mortality.
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http://dx.doi.org/10.1016/j.jacc.2011.06.041DOI Listing
September 2011